Improved Survival after Repeat Lung Transplantation at Cystic Fibrosis Care Centers

Abstract

Patients who undergo repeat lung transplantation (LT) are at high risk for perioperative complications. The complexity of their care requires specialized multidisciplinary collaboration, particularly among people with cystic fibrosis (CF). Since 1961, the Cystic Fibrosis Foundation has established a network of accredited Cystic Fibrosis Care Centers (CFCCs) dedicated to the treatment of people with CF, including LT. We hypothesize that patients undergoing repeat lung transplantation at CFCCs will have improved postoperative outcomes compared to those at non-CFCC centers. Using the Scientific Registry of Transplant Recipients, we identified adults with cystic fibrosis who received a repeat LT at a CFCC between 2005 and 2018. We compared clinical, demographic, and donor characteristics between groups. We then used multivariable Cox proportional hazards modeling to compare graft survival (defined as freedom from death, graft failure, or further retransplantation) in people with CF treated at CFCCs and non-CFCC centers during the study period. Our cohort included 303 people with CF who underwent repeat LT from 2005-2018. Median age at repeat transplantation was 30 years (SD 8) and 50% were male. During 877 person-years of observation, 159 (53%) patients died, 7 (2%) underwent another transplant, and 4 (1%) were lost to follow-up. In a multivariable model, graft survival was significantly improved at CFCC centers compared to non-CFCCs (adjusted HR 0.62, 95% CI 0.42-0.94). Median survival was 4.2 years among people treated at CFCCs and 2.8 years among those treated at non-CFCCs. In people with CF, repeat LT at CFCCs is associated with improved graft and overall survival compared to repeat LT at non-CFCCs. Treatment at a CFCC provides specialized, multidisciplinary care for people with CF and can lead to improved outcomes even among high acuity patient subgroups.